ovarian sex cord-stromal tumors
Definition: Ovarian sex cord-stromal tumors are a heterogeneous group of benign or malignant tumors that develop from the cells surrounding the oocytes, including the cells that produce ovarian hormones (ie, the nongerm cell and nonepithelial components of the gonads).
Ovarian sex cord-stromal tumors are rare, comprising 5 to 8 percent of all primary ovarian neoplasms.
In contrast to epithelial ovarian cancer, most patients with malignant sex cord-stromal tumors are diagnosed with early-stage disease, the tumors are generally considered to be low-grade malignancies, and there is no known association with the BRCA1 or BRCA2 germline mutations and genetic predisposition to breast and ovarian cancer.
Types
ovarian granulosa-stromal cell tumors
- ovarian adult granulosa cell tumor (adult-type granulosa cell tumor)
- ovarian juvenile granulosa cell tumor (juvenile type granulosa cell tumor)
- ovarian thecoma-fibroma group (fibroma-thecoma tumor group)
- ovarian fibroma and ovarian cellular fibroma
- ovarian fibrosarcoma
- ovarian stromal tumor with minor sex cord elements
- sclerosing stromal tumor
- signet-ring stromal tumor
ovarian Sertoli-stromal cell tumors
- ovarian Sertoli-Leydig cell tumor group
- ovarian Sertoli-Leydig cell tumor with heterologous elements
- retiform Sertoli-Leydig cell tumor and variant with retiform elements
- ovarian Sertoli cell tumor (SCT)
- ovarian large cell calcifying Sertoli cell tumor (LCCSCT)
- stromal-Leydig cell tumor
- sex cord-stromal tumors of mixed or unclassified cell types
- sex cord tumor with annular tubules
- gynandroblastoma
- steroid cell tumors
- steroid cell tumors, NOS (not otherwise specified)
- stromal luteoma
- ovarian Leydig cell tumor
- ovarian hilus cell tumor
- Leydig cell tumor, non-hilar type
ovarian mixed sex cord-stromal tumors
ovarian unclassified sex cord-stromal tumors
See also
Tumors
- testicular tumors
Links
Histopathology images on Medscape
References
Sex cord stromal tumors of the ovary in children: A clinicopathological report from the Italian TREP project. Cecchetto G, Ferrari A, Bernini G, Alaggio R, Collini P, Virgone C, Terenziani M, Dall’igna P, Cozza R, Conte M, Bisogno G. Pediatr Blood Cancer. 2011 Jul 1;56(7):1062-7. PMID: #21488154#
Tsuji T, Catasus L, Prat J. Is loss of heterozygosity at 9q22.3 (PTCH gene) and 19p13.3 (STK11 gene) involved in the pathogenesis of ovarian stromal tumors? Hum Pathol. 2005 Jul;36(7):792-6. PMID: #16084949#
Miettinen M, Wahlstrom T, Virtanen I, Talerman A, Astengo-Osuna C. Cellular differentiation in ovarian sex-cord-stromal and germ-cell tumors studied with antibodies to intermediate-filament proteins. Am J Surg Pathol. 1985 Sep;9(9):640-51. PMID: #2996374#